2009 Southern Regional Meeting Abstracts

Purpose of Study: Case report for the prompt recognition and treatment of a fatal complication.
Methods Used: Case report
Summary of Results: We report a case of retinoic acid syndrome (RAS), an oncologic emergency that can arise as a result of treatment of acute myelocytic leukemia (AML) with all-trans retinoic acid (ATRA). RAS is a life threatening condition that requires prompt diagnosis and treatment in order to avoid significant morbidity and mortality. It is crucial for a general internist to be familiar with the clinical characteristics and appropriate treatment of this uncommonly seen disorder in a medical ward.
A 36 year old man developed fatigue and cough followed by a fever. He was treated with levofloxacin for pneumonia. Blood work in the emergency department revealed acute myelocytic leukemia. Patient was started on retinoic acid and idarubicin for the treatment of AML. He developed fevers again and complained of non pleuritic chest pain one week later. Computed tomography (CT) chest showed interval increase in the extent of the bilateral diffuse and patchy infiltrates. Two-dimensional echocardiogram showed small pericardial effusion. Electrocardiogram (EKG) was consistent with acute pericarditis. He had no previous history of cardiac disease. Retinoic acid syndrome (RAS) was diagnosed due to the aggregation of symptoms such as fever, pulmonary infiltrates, pericardial effusion and respiratory distress. Patients should have three of the seven signs fever, respiratory distress, pulmonary infiltrates, weight gain, hypotension, renal failure pleural or pericardial effusions for the diagnosis of RAS according to the literature. Retinoic acid treatment was discontinued. High dose intravenous dexamethasone therapy was initiated with significant improvement in this patient’s signs and symptoms proven with repeat CT scan of the chest within a few days.
Randomized clinical trials are not available for management guidelines but case series have shown benefit with high dose dexamethasone treatment. It is also questionable whether discontinuation of ATRA after the development of symptoms has any benefits.
Conclusions: Nevertheless, it is important for a general practitioner to learn about this potentially fatal condition.